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Progressive arthropathy in the course of immune tolerance induction failure in a child with hemophilia A and FVIII inhibitor – a case report
Affiliations- Department of Paediatrics, Haematology and Oncology, Medical University of Warsaw, Warsaw, Poland
- Department of Radiology, University of Warmia and Mazury, Olsztyn, Poland
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Abstract
We present the case of an 18.5-year-old boy with hemophilia A and FVIII inhibitor detected at the age of 8 months. No persistent inhibitor eradication was achieved, despite three attempts of immune tolerance induction (ITI) with temporary inhibitor resolution and subsequent administration of 11 doses of rituximab. In spite of hemostatic treatment, rehabilitation, and radiosynovectomy, the patient suffered from recurrent bleeding episodes into almost all major joints, which resulted in the development of progressive hemophilic arthropathy. Currently, due to high frequency of bleeding episodes, the patient is being treated prophylactically and receives prophylaxis with activated prothrombin complex concentrate (aPCC).
Abstract
We present the case of an 18.5-year-old boy with hemophilia A and FVIII inhibitor detected at the age of 8 months. No persistent inhibitor eradication was achieved, despite three attempts of immune tolerance induction (ITI) with temporary inhibitor resolution and subsequent administration of 11 doses of rituximab. In spite of hemostatic treatment, rehabilitation, and radiosynovectomy, the patient suffered from recurrent bleeding episodes into almost all major joints, which resulted in the development of progressive hemophilic arthropathy. Currently, due to high frequency of bleeding episodes, the patient is being treated prophylactically and receives prophylaxis with activated prothrombin complex concentrate (aPCC).
Keywords
hemophilia A; factor VIII inhibitor; ITI; bypassing agents
About this articleTitle
Progressive arthropathy in the course of immune tolerance induction failure in a child with hemophilia A and FVIII inhibitor – a case report
Journal
Issue
Pages
81-84
Published online
2019-08-23
Page views
133
Article views/downloads
132
DOI
10.2478/ahp-2019-0013
Bibliographic record
Acta Haematol Pol 2019;50(2):81-84.
Keywords
hemophilia A
factor VIII inhibitor
ITI
bypassing agents
Authors
Paweł Łaguna
Anna Klukowska
Jarosław Ćwikła
Michał Matysiak